Brainstem glioma

Brainstem gliomas consist of a heterogeneous group which vary greatly in histology and prognosis.

It should be noted that if not otherwise specified the term brainstem glioma usually refers to the most common histology, the diffuse brainstem glioma, and in children is most likely a diffuse midline glioma H3 K27M–mutant

Brainstem gliomas account for ~25% of all posterior fossa tumours and are most common in children between 7 and 9 years of age 5. There is no recognised gender or racial predilection.

Brainstem gliomas are also recognised in adults, although they are rare accounting for only 2% of adult brain tumours 7. They typically occur in younger adults (third and fourth decade) and tend to be of low grade (WHO I or II) 7.

Although the exact presentation will vary according to location and size of the tumour, in general patients will exhibit a combination of  4:

The duration of symptoms is usually much shorter in diffuse gliomas, in which the history is typically very short (a few days) 4. Additionally, diffuse gliomas more frequently have multiple cranial nerve palsies.

Histology

Recognised histological types include:

Classification

The most frequently used classification system is to divide these tumours into four types 5:

  1. diffuse brainstem glioma
  2. focal brainstem glioma
  3. (dorsally) exophytic
  4. cervicomedullary
    • probably an artificial group made up of the downward extension of true brainstem gliomas or upward extension of upper cervical cord intramedullary spinal cord tumours 5
Associations
Location

As a general rule mesencephalic tumours tend to be of a lower grade than those in the pons and medulla 3.

  • pontine
  • mesencephalic
  • medullary
    • least common location
    • includes focal dorsally exophytic, focal, diffuse and cervicomedullary junction variants
    • cervicomedullary junction tumours usually represent upper cervical tumours extending superiorly
    • most common location for NF1 associated tumours
MRI

MRI is the imaging modality of choice. The appearance will vary with the tumour type, thus please refer to individual articles. 

DSA/angiography

May show anterior displacement of the basilar artery.

Again, both treatment and prognosis are significantly influenced by tumour type, morphology and location. Radiation is a key part of treatment.

As a general rule, dorsal exophytic tumours and cervicomedullary tumours tend to do best with surgery, whereas surgery has no role in the management of diffuse brainstem gliomas

Prognosis
  • diffuse
    • terrible prognosis
    • 90-100% patients die within 2 years of diagnosis 6
  • focal (tectal glioma)
    • excellent long-term survival with CSF shunting (essentially benign lesions)
  • focal (other)
    • good long-term prognosis with surgery
  • (dorsally) exophytic tumours
    • good long-term prognosis with surgery​
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Article information

rID: 5736
Section: Pathology
Synonyms or Alternate Spellings:
  • Brainstem gliomas
  • Brainstem glioma (general)
  • Brainstem gliomata

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